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doi:10.1016/j.jacc.2011.04.0142011;58;337-350 J. Am. Coll. Cardiol.

Kathleen Stergiopoulos, Elaine Shiang, and Travis BenchPregnancy in Patients With Pre-Existing Cardiomyopathies

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STATE-OF-THE-ART PAPER

Pregnancy in Patients With

Pre-Existing CardiomyopathiesKathleen Stergiopoulos, MD, PHD, Elaine Shiang, BA, Travis Bench, MD

Stony Brook, New York

To varying extents, women with pre-existing cardiomyopathies have a limited cardiovascular reserve. The hemo-dynamic challenges of pregnancy, labor, and delivery pose unique risks to this group of patients, which can re-sult in clinical decompensation with overt heart failure, arrhythmias, and rarely, maternal death. A multidisci-plinary team approach and a controlled delivery are crucial to adequate management of patients withunderlying heart disease. Pre-conception planning and risk assessment are essential, and proper counselingshould be offered to expectant mothers with regard to both the risks that pregnancy poses and the implications

for future offspring. In this article, we will review the hemodynamic stressors that pregnancy places upon womenwith pre-existing cardiomyopathies and risk assessment and discuss what evidence exists with regard to themanagement of 2 forms of cardiomyopathy during pregnancy, labor, and delivery: dilated and hypertrophiccardiomyopathy. (J Am Coll Cardiol 2011;58:337–50) © 2011 by the American College of Cardiology Foundation

Hemodynamic changes that occur during pregnancy chal-lenge the functional adaptability of the cardiovascular sys-tem in patients with pre-existing cardiomyopathies. Thegreater metabolic needs of pregnancy are met by changes inblood volume, peripheral vascular resistance, and myocardialfunction. In women with underlying cardiac disease, how-

ever, the demands of pregnancy pose additional stressorsthat can lead to decompensation, arrhythmias, and rarely,maternal death (1). Although heart disease is present in0.5% to 1% of all pregnant women, data from the UnitedKingdom suggest that a cardiac etiology is the most com-mon cause of death among pregnant women in the devel-oped world (2,3). In a recent study that examined theoutcomes of pregnant women with dilated cardiomyopathy,cardiac complications were common, accounting for ap-proximately 39% of pregnancies (4). Heart failure was themost common complication, which typically occurred late inthe pregnancy or post-partum. Additionally, pregnancy in

women with pre-existing heart disease is associated withconsiderable morbidity and mortality (5), and although theincidence has been relatively constant over the past 2decades, a slight increase has been noted recently. In thispaper, we review the hemodynamic stressors that pregnancy places upon women with heart disease and risk assessmentand discuss what evidence exists with regard to the man-agement of dilated and hypertrophic cardiomyopathy

(HCM) during pregnancy, labor, and delivery. Pregnancy-related cardiomyopathy, or peripartum cardiomyopathy, willnot be discussed, because this subject has been recently reviewed (6–10).

General ConsiderationsHemodynamic changes during pregnancy. Dramaticchanges occur to the cardiovascular system during preg-nancy. Initially, marked increases in circulating blood vol-ume are met with an increase in stroke volume and a 15% to20% increase in heart rate. The net effect is a 30% to 50%increase in cardiac output by the end of the rst trimester,an effect that peaks between the second and third trimesters(8,11). Another important consideration is the maturationof a placental circulation, which provides a substantialreduction in systemic vascular resistance. During the thirdtrimester, preload reduction might occur due to compres-sion of the inferior vena cava (IVC) by the gravid uterus,thus reducing cardiac output. Increases in cardiac outputand intravascular volume allow 1 cardiac pump to feed bothmaternal and fetal tissues. It is indisputable that blood volume increases in pregnancy, but studies differ on when volume expansion levels off, if at all (1). Increases in blood volume enhance left ventricular end-diastolic volume, whichpeaks during the third trimester. This increased preload isthought to be due, in part, to an estrogenic effect, whichcreates higher circulating renin levels and greater sodiumand water retention. Alternatively, hormones such as pro-lactin, human placental lactogen, prostaglandins, andgrowth hormone have also been implicated. During theearly stages of pregnancy, increases in stroke volume are

From the Division of Cardiovascular Disease, Department of Internal Medicine,Stony Brook University Medical Center, Stony Brook, New York. The authors have

reported that they have no relationships to disclose.Manuscript received January 14, 2011; revised manuscript received March 24,2011, accepted April 12, 2011.

Journal of the American College of Cardiology Vol. 58, No. 4, 2011© 2011 by the American College of Cardiology Foundation ISSN 0735-1097/$36.00Published by Elsevier Inc. doi:10.1016/j.jacc.2011.04.014




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largely responsible for the ob-served increase in cardiac output, whereas later in pregnancy, anincreased heart rate accounts forthese changes. These physiologiceffects are facilitated by the de-creased systemic vascular resis-tance created by the placenta, aphenomenon that continues untilthe 32nd week of pregnancy when afterload begins to riseagain. Besides the nding of el-evated renin levels in the settingof an expanded intravascular vol-ume, the integration of the renaland cardiovascular systems is alsoevident by the release of atrialnatriuretic peptide and B-typenatriuretic peptide (BNP) in re-sponse to atrial and ventriculardistension, respectively (12).

Hemodynamic changes during labor and delivery. Thecardiovascular system of women with heart disease is limitedin its ability to accommodate the demands of pregnancy. These limitations become more evident during labor anddelivery, where several changes in the circulatory systemcould result in hemodynamic decompensation (1). There isa catecholamine-induced increase in heart rate and stroke volume due to pain and anxiety. During the peripartumperiod, there can be an increase of cardiac output of up to31% and approximately 50% in the second stage of labor. Abrupt changes in uid balance result from a lack of IVCcompression as well as the redistribution of blood from thelower limbs, particularly during uterine contractions. Thisrapid increase in preload can result in pulmonary congestionand clinical heart failure. Some of this intravascular volumeis lost at delivery, where variable blood loss will occur—approximately 500 ml with a normal vaginal delivery, and1,000 ml for a routine cesarean section. Further alterationsin the hemodynamic status occur most commonly withinthe rst 12 to 24 h post-partum. Within the rst hour of delivery, cardiac output might continue to increase to asmuch as 80% above pre-labor values due to the relief of IVCcompression and potentially rapid autotransfusion from theplacenta (13). Moreover, further uctuations in hemody-namic status can be due to the loss of the low resistanceplacenta and a relative increase in systemic vascular resis-tance as well as the mobilization of dependent edema andinterstitial uid. The use of anesthesia and analgesia cancause hypotension as a result of venous pooling and de-creased systemic vascular resistance. Therefore, women withpre-existing cardiomyopathies might be at high risk forperipartum complications, due to the inability to accommo-date increased cardiac output.Pre-conception risk assessment and counseling. Women with cardiac disease require a complete pre-conception

evaluation and counseling to risk-stratify the maternal andfetal risks of pregnancy ( Table 1). As such, appropriateevaluations can take place without putting the fetus at risk. A detailed history and physical examination, assessment of functional capacity and New York Heart Association(NYHA) functional class, and a 12-lead electrocardiogramare essential. Echocardiography is indicated in women witha history of valvular or congenital heart disease, signicantdyspnea or any symptoms, any signs of heart failure, any systolic murmur grade II, or any diastolic murmur. Inaddition, the etiology and degree of valvular regurgitationand/or stenosis, degree of pulmonary hypertension, and—if present—aortic root dilation can be quantied. Importantly,the left ventricular or systemic ventricular systolic functioncan also be determined. In certain congenital heart diseasepatients, assessment of the right heart size and function canbe achieved most accurately with cardiac magnetic reso-nance imaging. Exercise stress testing can be useful toquantify the functional capacity of a patient if the history of the patient is unclear. However, this should ideally beperformed before pregnancy. Poor functional status hasbeen previously identied to be associated with maternal orfetal complications (14). Functional capacity might be animportant predictor of the ability to tolerate a pregnancy,regardless of the underlying lesion. In a recent study examining pregnancy outcomes in women with congenitalheart disease, an abnormal chronotropic response correlated with adverse pregnancy outcomes and could be consideredin rening risk stratication schemes (15).

Women at particularly high risk include those with mechan-ical heart valves, Eisenmenger’s syndrome, Marfan syndrome with aortopathy (aortic root 40 mm), and cardiomyopathy with reduced systemic ventricular function (left ventricularejection fraction [LVEF] 40%) or a history of peripartumcardiomyopathy. In these women, pregnancy might be atprohibitive maternal risk and counseling with regard to avoid-ance of pregnancy might be ideal. Among pregnant women with known cardiac disease, poor prognostic factors includeany of the following: prior cardiac events, prior arrhythmias, anNYHA functional class II, peripheral cyanosis, signicant valvular or outow tract obstruction, and systemic ventriculardysfunction with LVEF 40% (16–18). Moreover, when 1 ormore of these features are combined, the risk is more thanadditive (16–18). As such, a risk score has been developed by Siuet al. to identify predictors associated with the developmentof unfavorable cardiac events in pregnant women with heartdisease and might be used to allow for the establishment of aplan of management for the antepartum, peripartum, andpost-partum periods. If a woman has any 1 of the aforemen-tioned poor prognostic factors, the estimated/expected risk is27%, whereas if she has 1 risk factor, the risk rises to 75%. Women at elevated risk for adverse events should be managedby a multidisciplinary team at a tertiary care center equipped with the expertise to handle high-risk pregnancies (19). As partof a complete risk assessment, plans for monitoring, type of delivery, and anesthetic concerns should be addressed.

Abbreviationsand Acronyms

BNP B-type natriuretic

peptide

COC combined hormonal

contraceptive

HCM hypertrophic

cardiomyopathy

IVC inferior vena cava

LVEF left ventricular

ejection fraction

LVOT left ventricular

outow tract

NT-proBNP N-terminal

pro–B-type natriuretic

peptide

NYHA New York Heart

Association

WHO World HealthOrganization

338 Stergiopoulos et al. JACC Vol. 58, No. 4, 2011Cardiomyopathy and Pregnancy July 19, 2011:337–50




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Pre-conception evaluation, risk assessment, and propercounseling are essential (20). Women with congenital heartdisease, in particular, should be aware of adverse fetal andmaternal outcomes as well as the genetic susceptibility of heroffspring to heart disease. A study of women with congenitalheart disease found that many of these women could notrecall whether they were ever properly counseled by a healthcare provider or whether they were ever informed of anincreased risk for maternal complications (21). Strict pre-natal care and early risk stratication are fundamentalmeasures to improve the prognosis of pregnancy in women with heart disease.Contraceptive choices in women with heart disease. In women with heart disease, choice of contraception requiresconsideration of pregnancy risk, available contraception op-tions as well as their risks and benets, failure rates, under-standing the consequences of unplanned pregnancy, and thepreferences of the woman. Cardiologists are responsible,

beyond risk assessment, for educating women about safecontraceptive options available as they relate to their cardiaccondition. Interestingly, the current American College of Cardiology/American Heart Association guidelines for themanagement of adults with congenital heart disease suggeststhat it is the duty of the cardiologist to provide advice regardinginformed decisions on contraception (22). Likewise, this rec-ommendation could be extended to all women of childbearingage with heart disease. Moreover, understanding the risk of pregnancy is important to determine which type of contracep-tion is required. Notably for example, for women with Eisen-menger’s syndrome, in which pregnancy is contraindicated dueto prohibitively high maternal risks, permanent forms of sterilization can be considered.

Contraceptive options include: 1) combined hormonal contra-ceptives (COCs; estrogen/progestin formulations); 2) progestin-only formulations; 3), intrauterine devices; 4) barrier methods;and 5) sterilization/permanent forms of contraception. Thereare limited published reports addressing the issue of contra-ceptive use in women with heart disease (23,24). The mostcomprehensive guidance comes from a British working groupthat developed guidelines for the use of COCs in women withheart disease with the World Health Organization (WHO)format (23,25–27). Although both estrogen and progestinshave adverse cardiac effects, the most clinically important arethose of estrogens that cause thromboembolic events andhypertension. In this schema, there are 4 classications for theuse of COCs. The WHO Class 1 includes conditions wherethere is no restriction on the use of COCs, such as simplecongenital lesions (successfully repaired atrial or ventricularshunts), and bicuspid aortic valve. The WHO Class 2 suggeststhat the benets outweigh the risks of the use of COCs andincludes conditions such as HCM without evidence of atrialarrhythmias and past cardiomyopathy that has fully recovered.Class 3 suggests that the risk of the use of COCs likely outweighs its benets and includes bileaet mechanical pros-thesis in the mitral and aortic position while taking warfarinand atrial brillation or utter while taking warfarin. Class 4represents the highest-risk group for the use of combinedestrogen and progestins and includes conditions such as cya-notic heart disease, prior left ventricular systolic dysfunction(LVEF 30%), and coronary artery disease or arteritis. Adetailed description of methods ofcontraceptionandWHOClass1 to 4 conditions can be found elsewhere (23).

In women with severe systemic ventricular dysfunction,maternal complication rates of pregnancy are high, and inmany cases, pregnancy is contraindicated. However, due

Pre-Conception Evaluation and Risk AssessmentTable 1 Pre-Conception Evaluation and Risk Assessment

Thorough history of cardiac symptoms and physical examination

12-lead electrocardiogram

Baseline exercise tolerance and functional class (exercise testing if needed)

Baseline echocardiogram

Assessment of ventricular function (right and left)Assessment of pulmonary artery pressure

Presence and degree of valvular dysfunction

Assessment of stability of cardiac hemodynamic status over time

Effective contraception until pregnancy desired

Adjust medications to prevent adverse fetal events

Genetics referral for patients with heritable cardiac lesion

Risk Stratication for Pregnant Patients With Cardiac Disease: High Risk for Adverse Maternal and Fetal Outcomes

Any prior cardiac event or arrhythmia

NYHA functional class II

Systemic ventricular dysfunction (ejection fraction 40%)

Pulmonary hypertension (PA systolic pressure 50% systemic pressure), whether isolated or associated with severe valve disease

Left heart obstruction

Severe aortic stenosis (valve area 1 cm2

, Doppler jet velocity 4 m/s)Symptomatic or severe mitral stenosis

Severe aortic or mitral regurgitation with NYHA functional class III or IV symptoms

NYHA New York Heart Association; PA pulmonary artery.

339JACC Vol. 58, No. 4, 2011 Stergiopoulos et al.

July 19, 2011:337–50 Cardiomyopathy and Pregnancy




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to the potential for thromboembolic complications, com-bined hormonal contraceptives in the form of pills,transdermal patches, or vaginal rings are not recom-mended. In this group, progesterone-only forms of contra-ception and intrauterine devices are appropriate options. Although progestin-only forms of contraception are oftensuitable for women with severe cardiomyopathies, the oralform or the “mini-pill” has a high failure rate (5% to 10%)in the rst year of use. Therefore, injectable or implantable versions of progestin-only formulations might be a betterchoice. In contrast, for women with HCM, the WHOclassication of combined hormonal contraception is gen-erally considered class 2, which is broadly usable.

Counseling women with heart disease with regard tocontraception is being poorly done or not at all (21,28). Ina survey of women with congenital heart disease, it wasnoted that only one-half of the women surveyed hadrecalled receiving specic information about contraceptionfrom a nurse or physician (21). In another recent study of women with congenital heart disease, many women do notuse adequate methods of birth control (29). Consequently,there is room for improvement in this area.Evaluation of heart failure in pregnancy. The normalphysical examination in pregnancy can often mimic disease.Increased plasma volume might result in a systolic ow murmur, which can be heard in most normal pregnantpatients. This murmur is usually systolic and soft (usually grade II/VI). Moreover, distended or mildly increasedneck veins, mild lower-extremity edema, and tachycardia arenormal, commonly observed ndings. In addition, commoncomplaints of a normal pregnancy such as palpitations,fatigue, decreased exercise tolerance, and orthopnea canoften be identical to those with occult or overt heart failure.During pregnancy, some patients might require careful,frequent follow-up evaluations, which might increase infrequency according to symptoms or severity of disease.Repeat echocardiographic imaging—perhaps as often asonce/trimester or sooner as clinically indicated—can behelpful, because many symptoms of a normal pregnancy canmimic heart failure in the antepartum period. Due to aninability to increase cardiac output in the setting of an ex-panded intravascular blood volume, patients with an underly-ing cardiomyopathy often develop clinical heart failure, andtherefore ongoing surveillance is valuable. Patients with left ventricular dysfunction might require restriction of activities,inpatient observation before delivery, and initiation or altera-tion of medical therapy. Value of biomarkers in pregnancy. Limited data areavailable on the value of serum BNP or N-terminal pro–B-type natriuretic peptide (NT-proBNP) levels in pregnancy.In a small series of normal pregnant women, there were nosignicant differences in the mean BNP levels at variousstages of pregnancy and the post-partum period (30–32).However, pregnant women had higher BNP levels that,although within the normal range, were approximately twiceas high as nonpregnant levels. In women with pre-

eclampsia, levels of circulating atrial natriuretic and BNPare directly related to changes in left ventricular mass and volume (33). Now widely used to screen for ventriculardysfunction, BNP values in women with severe pre-eclampsia were found to be signicantly higher than thosefrom a normal study population, reecting greater ventric-ular wall stress associated with this condition (31). More-over, in a small series of pre-eclamptic women, diastolic left ventricular function was impaired, and levels of NT-proBNP were found to be increased, compared with that innormal pregnancies (34). In a more recent study, BNP values were higher in pregnant women with heart diseasethan without (35). In addition, among women with clinicalevents, all were found to have elevated BNP values, whereasno events occurred among women with a BNP value100pg/ml. Thus, BNP had a negative predictive value of 100%for identifying events during pregnancy in this small series.However, a subset of women with elevated BNP values wasidentied without any clinical events, a nding of unclearsignicance. Certainly, most healthy pregnant women hadlow and stable concentrations of BNP throughout theirpregnancy, suggesting that these women are able to com-pensate for the hemodynamic load of pregnancy. Althoughlimited clinical data are available, measuring BNP or NT-proBNP levels seems to have clinical utility when thediagnosis of heart failure is in question (35).Medical management of heart failure in pregnancy. Thegoals of medical therapy in chronic heart failure patientsduring pregnancy are similar to those of nonpregnantpatients (36). Whenever possible, the continuation of chronic therapies that improve long-term outcomes in women with heart failure remains an important consider-ation. One important exception is that angiotensin-converting enzyme inhibitors and angiotensin receptorblockers are contraindicated in women who are pregnant ormight become pregnant, due to the teratogenic effects onthe fetal kidneys ( Table 2) (8,37). Additionally, aldosteroneantagonists should not be used in pregnant women. Provid-ers should discontinue these medications ideally in women who are planning to become pregnant or as soon aspregnancy is conrmed. Women who are planning tobecome pregnant must weigh the risks of discontinuation of drugs that prolong survival in the setting of left ventriculardysfunction against the potential for teratogenicity, which ispresent throughout pregnancy, even during the rst trimes-ter (38). Women taking beta-blockers for the treatment of chronic heart failure should continue them during preg-nancy, even if asymptomatic. Vasodilator therapy, whennecessary, can be achieved with hydralazine or amlodipine,because there are published data supporting safety of thesemedications in pregnancy particularly in the setting of hypertension (39). Sodium restriction is recommended forall patients, whereas loop diuretics are indicated for thesymptomatic relief of signicant peripheral edema or pul-monary congestion. Moreover, digoxin can be added orcontinued during pregnancy for the symptomatic relief of





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heart failure symptoms, after beta-blockers and vasodilatorshave been maximized.

In the setting of acute decompensation of heart failure,

particularly if severe, therapies are directed similarly tononpregnant women. Intravenous diuretics and intravenous vasodilator therapy with nitroglycerin can be used safely.Right heart catheterization might be necessary, if thediagnosis of heart failure is in question on the basis of physical examination or when large shifts in hemodynamicstatus are anticipated (as in labor and delivery). Right heartcatheterization is uncommonly used in the setting of pregnancy.

Dilated Cardiomyopathy

Dilated cardiomyopathy is dened as reduced left ventricularsystolic function in the absence of coronary, valvular, congen-ital, or systemic disease known to cause myocardial dysfunction

(40). Affected patients might even have impaired systolicfunction in the absence of any symptoms. Currently, dilatedcardiomyopathy is responsible for approximately 10,000 deaths

and 46,000 hospital stays each year in the United States and isthe most common indication for cardiac transplantation (41). Although there are several known causes of dilated cardiomy-opathy ( Table 3), the etiology remains undened in approxi-mately 50% of cases (42) (Figs. 1 and 2).

Women with dilated cardiomyopathy have tradition-ally been advised to avoid pregnancy. This dictum islargely based on data derived from studies in patients with peripartum cardiomyopathy and the observation of poor peripartum maternal and fetal outcomes in thisgroup (16–18). Dilated cardiomyopathy has been associ-ated with A-type lamin gene defects, which are associated with a high rate of heart failure and life-threateningarrhythmias, as predicted by NYHA functional class and

Medical Management of Chronic Heart Failure in PregnancyTable 2 Medical Management of Chronic Heart Failure in Pregnancy

Drug/Class Purpose Comment

Diuretics

Furosemide Generally reserved for treatment of pulmonary edema Can result in uteroplacental hypoperfusion

Use of lowest possible dos e Contra-indicated in settings in which uteroplacental

hypoperfusion is already reduced (IUGR,pre-eclampsia)

FDA class C*

Digoxin Not considered rst-line therapy for heart failure innonpregnant patients

No improvement in mortalityConsidered useful in pregnancy, given limitations of

medical armamentarium

Generally considered safeUseful in treatment of persistent symptoms,

despite standard therapyFDA class C

Vasodilators

Hydralazine Commonly used oral antihypertensive agent in pregnancyCan be substituted for ACE inhibitor during pregnancy

Demonstrated efcacy in hypertensionRisk of hypotensionPregnancy already reduces SVRAvoid large or precipitous decreases in blood

pressureFDA class C

ACE inhibitors/ARB Proven benet in treatment of chronic heart f ailure innonpregnant patients

Contraindicated throughout pregnancy due toteratogenic effects. Associated witholigohydramnios, neonatal death secondary torenal failure, renal agenesis.

FDA class C for rst trimester; class D for secondand third trimesters

Amlodipine Alternative to ACE inhibitor in pregnancy Can be used with hydralazine if neededFDA class C

Nitrates Might be used to treat decompensated heart failure FDA class C

Beta-blockers

Carvedilol, labetalol, metoprolol, atenolol Essential component to chronic heart failure therapyAgents that are beta-1 selective are preferableBeta-blockers should be continued throughout pregnancy

Generally safe and effective in pregnancyCan cause IUGRInfants born to mothers taking beta-blockers should

be observed for at least 72 h after birthFDA class C

Aldosterone antagonistsSpironolactone, epleronone Prolong survival in selected heart fai lure patients

Not routinely used in pregnancyNo data to support safety in pregnancyFDA class D

Warfarin Risk/benet ratio needs to be discussed with the patientfor treatment and prophylactic anticoagulation insevere left ventricular dysfunction

First trimester teratogenesisDosing is complicated in pregnancyFDA class X

*U.S. Food and Drug Administration (FDA) class: A (controlled studies show no risk), B (no evidence of human risk in controlled studies), C (risk cannot be ruled out), D (positive evidence of risk),X (contraindicated in pregnancy).

ACE angiotensin converting enzyme; ARB angiotensin receptor blocker; IUGR intrauterine growth retardation; SVR systemic vascular resistance.






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the type of mutation (43). The increase in intravascular volume and cardiac output during pregnancy leads to agreater risk for complications in women with dilatedcardiomyopathy, particularly in the third trimester duringa period of maximal hemodynamic burden. A history of cardiac events including previous episodes of heart fail-ure, atrial brillation or utter, transient ischemic attack,or a history of cardiac events before pregnancy was highly predictive of pregnancy-related cardiac outcomes (16).Heart failure, arrhythmias, and stroke were more com-mon in women whose LVEF was 40% (18).

There is insufcient evidence relating to specic etiologicfactors to determine whether pregnancy might be better-tolerated in certain cardiomyopathies compared with others.In a small retrospective cohort study comparing patients with peripartum cardiomyopathy with patients with dilatedcardiomyopathy, those with peripartum cardiomyopathy had uniformly worse outcomes (44). These included 3maternal deaths and 4 heart transplantations in the peripar-tum group, compared with 1 transplantation in the dilatedcardiomyopathy group. None of the dilated cardiomyopathy patients had a signicant decline in cardiac status. Incontrast, a study of 26 women from Brazil suggested thatcardiac complications were higher in the idiopathic cardio-

myopathy cohort when compared with those with persistentleft ventricular dysfunction due to peripartum cardiomyop-athy (45). A study from Haiti identied 99 women withperipartum cardiomyopathy, 15 of whom had subsequentpregnancies. One-half of these women experienced worsen-ing heart failure and long-term systolic dysfunction (46).However, all 3 studies suffer from retrospective design issuesand selection bias.

Maternal cardiac, obstetric, and fetal outcomes in women with dilated cardiomyopathy were examined in a recentstudy by Grewal et al. (4). Thirty-six pregnancies in 32 women were evaluated as part of a larger prospective study on outcomes in women with heart disease. Age-matchedoutcomes were compared with nonpregnant women withunderlying cardiomyopathies. Notably, 39% of the preg-nancies were complicated by at least 1 maternal cardiacevent. In the multivariate analysis, moderate or severe LV dysfunction and/or NYHA functional class III or IV werethe main determinants of adverse maternal cardiac out-comes. In particular, the subset of women with moderate orsevere left ventricular dysfunction had the worst 16-monthevent-free survival. In women without moderate or severeLV systolic dysfunction, NYHA functional class III or IV,and/or previous cardiac event, no adverse events wereobserved. However, if any 1 of these 3 factors was present,the risk of an adverse event was 64%. Moreover, theinvestigators found that the event rate for adverse neonatalevents was highest among women with both obstetric andcardiac risk factors combined (43%).

Patients with known left ventricular dysfunction (LVEF 40%) are at high risk for adverse maternal and fetal events

and should be advised against pregnancy. Of particularconcern, women with prior peripartum cardiomyopathy in whom left ventricular function has returned to normal(LVEF 50%) still remain at signicant risk for morbidity and mortality with a subsequent pregnancy (47). In addi-tion, patients with class III or IV symptoms and moderate-to-severe left ventricular dysfunction in the rst or secondtrimester can be considered for termination of pregnancy.Management of labor and delivery in dilated cardio-myopathy patients. A multidisciplinary team is crucial toadequate management of patients at the time of labor anddelivery. Consultation among the obstetrician, obstetricalanesthesiologist, and the cardiologist is recommended be-fore initiation of labor and delivery. In addition, patients with heart failure or underlying cardiomyopathy should bemonitored carefully throughout labor and delivery as well asin the early post-partum period, when hemodynamic de-compensation is most likely to occur. This includes con-tinuous maternal electrocardiographic monitoring andnoninvasive blood pressure monitoring. Invasive centralmonitoring such as right heart catheterization and arterialline monitoring can be employed on an individual basis. Arterial line monitoring is considered helpful and low-risk.Right heart catheterization, although not required, might beneeded to optimize hemodynamic status when large shifts in

Etiology of Dilated CardiomyopathiesTable 3 Etiology of Dilated Cardiomyopathies

Idiopathic: 50%

Myocarditis: 9%

Viral cardiomyopathy

Chagas disease

Lyme diseaseHIV infection

Ischemic heart disease: 7%

Inltrative disease: 5%

Sarcoidosis

Peripartum cardiomyopathy: 4%

Hypertension: 4%

HIV infection: 4%

Connective tissue disease: 3%

Systemic lupus erythematosus

Substance abuse: 3%

Alcohol-related

Cocaine

Chemotherapy: 1%Adriamycin

Doxorubicin

Trastuzumab

Other: 10%

Stress-induced (Tako-tsubo) cardiomyopathy

Noncompaction

Hypertrophic cardiomyopathy

Tachycardia-mediated cardiomyopathy

Endocrine related (thyroid dysfunction, acromegaly, pheochromocytoma)

Inherited/familial cardiomyopathy

Obstructive sleep apnea

Adapted from Felker et al. ( 42 ).

HIV human immunodeciency virus.





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Figure 1 Example of Decline in Left Ventricular Systolic Function

A 28-year-old woman, with a history of viral cardiomyopathy with severely reduced left ventricular ejection fraction, presented at 21 weeks of gestation. Her medical history wascomplicated by asthma, obesity, presumed obstructive sleep apnea, diabetes, and active tobacco and drug abuse, and an implantable cardioverter-debrillator had been placedfor primary prevention of sudden cardiac death. She was considered, upon risk assessment, at prohibitively high risk for adverse maternal and fetal outcomes but adamantly refused termination of pregnancy despite extensive counseling. She was hospitalized for congestive heart failure at 26 weeks, which was managed with in-hospital bed rest andmedically with digoxin, carvedilol, hydralazine, and furosemide. She developed worsening heart failure and worsening left ventricular systolic function (left ventricular ejection frac-tion declined from 25% to 10%) at 28 weeks of gestation and was delivered by urgent cesarean section with an epidural and sedation and placement of a pulmonary artery catheter and arterial line. Of note, the wedge pressure of the patient was 28 mm Hg at the beginning of the procedure. Her post-partum course was complicated by a transientischemic attack. (A) Parasternal long-axis view demonstrating severe left ventricular dilation and tethering of the mitral leaets. (B) M-mode of the left ventricle demonstratingsevere left ventricular systolic dysfunction. In addition, she has a small-to-moderate posterior pericardial effusion without hemodynamic signicance, a common nding in preg-nancy. (C) Diastolic parameters of mitral inow E and A waves and severely reduced lateral tissue Doppler velocities, consistent with restrictive physiology and elevated lling

pressures, which progressed from pseudonormal diastolic function early in pregnancy.






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volume are anticipated, such as during a cesarean section or when there is evidence of clinical instability (19). Althoughno ofcial recommendations exist, right heart catheteriza-tion is occasionally used in pregnant women with severeclinical heart failure and/or severely reduced left ventricularsystolic function at the time of delivery.

The decision regarding the timing and mode of delivery is made on the basis of the hemodynamic status of thepatient ( Table 4). Early delivery is not required in allpatients with cardiomyopathies or heart failure. The deci-

sion is made on the basis of the failure of the patient torespond to medical therapy and the overall hemodynamicstatus of the patient. The issue of the timing of delivery forcritically ill pregnant women with heart failure requires acoordinated decision between the cardiologist, obstetrician,and anesthesiologist that balances the risks of continuing apregnancy to the mother and fetus versus the risk of delivery and how that delivery should take place. If the heart failureof the patient is refractory to medical therapy, delivery needsto be strongly considered. Because no ofcial recommenda-tions exist, an individualized approach is sought. Virtually all pregnant women with cardiac disease should expect anattempt at vaginal delivery, unless obstetric contraindica-tions exist. For women with pre-existing cardiac disease, a vaginal delivery poses less cardiac risk, because cesareandelivery is accompanied by approximately twice as muchblood loss. Patients who are considered stable from a cardiacperspective can be allowed to spontaneously progress

Figure 2 Cardiac Magnetic Resonance ImagingDemonstrating Morphological Noncompaction

Criteria include a maximal end-systolic ratio of noncompacted endocardialthickness to compacted epicardial layer thickness (N/C) 2 (here, seen mostprominently in the lateral wall of the left ventricle). Isolated noncompaction of the left ventricle can also present in the peripartum period as the rst episodeof heart failure, arrhythmia, or embolic event. Underlying noncompaction mightbe present in asymptomatic antepartum patents, who cannot tolerate thehemodynamic stress of pregnancy, resulting in clinical decompensation. Sucha presentation might be confused with peripartum cardiomyopathy, which canhave a similar clinical course. (A) 4-chamber view. (B) 2-chamber view. Bothviews demonstrate noncompacted myocardium.

Management During Labor and DeliveryTable 4 Management During Labor and Delivery

Decision regarding timing and mode of delivery

Management during labor and delivery and post-partum concerns

Short vaginal delivery with excellent anesthesia, with consideration ofassisted second stage of labor

Left lateral decubitus position

Cesarean section per obstetric indications

Invasive monitoring if needed (right heart catheterization, invasive arterialblood pressure monitoring)

Medical therapy optimization of loading conditions

Monitoring and treatment of pulmonary edema

Anesthetic choices for labor and delivery in the setting of heart failure

General anesthesia

Volatile agents include sevourane, isourance, and desurane, which candecrease SVR

Reserved for emergency situations

Rapid sequence induction can lead to cardiovascular instability

Mortality is highest at the time of induction and intubation

Regional anesthesia

Includes spinal, epidural, or combined spinal-epidural

Technique of choice in patients with heart failure and pregnancy for delivery

Offers afterload reduction and blunts hemodynamic response oflabor and delivery

Low concentration of bupivacaine and lipophilic opiates allow for

hemodynamic stabilitySedation

Can accompany regional techniques if needed

Agents such as propofol, midazolam, and fentanyl have been usedwithout fetal issues

Aspiration risk exists

Post-partum concerns

Consider treatment of severe anemia

Medical therapy to optimize loading conditions (treatment ofpulmonary edema)

Hemodynamic and telemetry monitoring for 12–24 h

Contraception or sterilization consideration

Future consideration of ICD*

*Indications for insertion of an implantable cardioverter-debrillator (ICD) would follow acceptedguidelines ( 48 ).

SVR systemic vascular resistance.





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through the various stages of labor. However, if there areconcerns about the functional adequacy of the heart andcirculation, labor can be induced under controlled condi-tions. The timing of induction can be individualized, takinginto account the cardiac status of the patient, inducibility of the cervix, and fetal lung maturity. From a practical point of view, it is useful to plan the induction so that delivery occursduring a time when all services are available. In general, along induction in a woman with an unfavorable cervixshould be avoided. Induction of labor in a patient with afavorable cervix usually requires only oxytocin administra-tion and articial rupture of membranes. An unfavorablecervix, however, should be treated with a prostaglandin Eanalogue. Even this should be done cautiously in women with underlying cardiomyopathies, because prostaglandinanalogues might be absorbed systemically, causing un- wanted hemodynamic consequences, including a decreasedsystemic vascular resistance and reex tachycardia (49). In arecent study of patients with dilated cardiomyopathy, mostdeliveries were vaginal (81%), and the most frequently usedform of anesthesia was epidural anesthesia (86%) (4).Indications for cesarean delivery were unrelated to pre-existing cardiac disease in any 1 of these patients. As wouldbe expected, fetal/neonatal event rates were higher in women with at least 1 obstetric risk factor, including ahistory of premature delivery or rupture of membranes, anincompetent cervix, or the need for cesarean delivery.Smoking, anticoagulation use, multiple gestation, and ma-ternal age 20 or 35 years were nonobstetric risk factorsthat were also associated with increased fetal/neonatalevents.

Anesthetic considerations in pregnant women withchronic or new onset heart failure require a specializedapproach and, when possible, should be planned in theantepartum period. Women with cardiomyopathy and/orevidence of clinical heart failure should not expect a trial of natural childbirth without the use of some form of anesthe-sia when a vaginal delivery is decided upon. The goal of theanesthetic agents is to blunt the physiologic increasedmetabolic demands and hemodynamic stress that normally accompanies labor and delivery. The goals of managementare mainly to avoid excessive anesthetic-induced myocardialdepression, maintain normovolemia, and minimize the in-herent sympathetic stimulation associated with labor (19). Acombination of intravenous opiates and lumbar epiduralanesthesia are highly effective to relieve pain during laborand delivery and is considered the technique of choice.Epidural anesthesia, if introduced slowly and carefully,produces changes in pre-load and afterload that can beadvantageous in the setting of reduced ventricular function.It provides excellent operative analgesia, thus limiting pain-induced elevations of sympathetic activity, but also reducesthe maternal urge to push (Valsalva maneuver). Addition-ally, the accompanying venodilation reduces venous return, which might also be favorable for those patients withevidence of volume overload. Decreases in systolic blood

pressure might require treatment with vasoactive agentsrather than intravenous uids. Alternatively, the use of general anesthesia incurs the risks of hemodynamic in-stability associated with systemic anesthetic administra-tion as well as adequate sedatives to tolerate endotrachealintubation.

Once in labor, women with cardiac disease should beplaced in a left lateral decubitus position to avoid IVCcompression by the gravid uterus. The obstetrician shouldallow the fetal head to descend to the perineum withoutmaternal assistance, an attempt to avoid the undesirablecirculatory effects of the Valsalva maneuver. The secondstage of labor can be shortened via assistance with low forceps or by vacuum extraction as needed. Throughout thisprocess, the clinical scenario should dictate the need toabandon further attempts at vaginal delivery and to proceed with cesarean delivery.Post-partum considerations. After delivery, uterine bleed-ing is controlled naturally by continued uterine contractionsfrom posterior pituitary oxytocin secretion. Synthetic oxy-tocin is often administered to augment these effects butshould be infused slowly to avoid hypotensive effects.Hemodynamic monitoring of the mother should continuefor at least 12 to 24 h after delivery, because volumeredistribution during this time period causes rapid intravas-cular volume shifts. If severe anemia is present, often relatedto dilutional anemia of pregnancy, exacerbated by blood lossat delivery, treatment could be considered with iron supple-mentation or blood transfusion in an effort to relievetachycardia and decrease myocardial work. Other post-partum considerations are listed on Table 4.

Although inciting events are difcult to dene, pregnancy seems to affect the natural history of dilated cardiomyopa-thy, particularly over the short term (4). Cardiac complica-tions such as worsening heart failure accompanied by worsening LVEF, arrhythmias, and cerebrovascular acci-dents were noted to occur most commonly in pregnant women studied late during their pregnancy as well as in therst 16 months post-partum. It is known that the thirdtrimester and early post-partum periods are times of maxi-mum hemodynamic change, supporting the concept thataccelerated changes in hemodynamic load can precipitatecardiac decompensation. But even beyond hemodynamicuctuations, a transient decline in left ventricular contrac-tility during pregnancy has even been described, whichmight be even more important in patients with poor cardiacreserve (50). It also seems that this decline in left ventricularsystolic function might not be reversible (an example of which is shown in Fig. 1). An important issue seems to bethe continuation of optimal medical therapy for heart failureduring pregnancy, which is not possible secondary to theteratogenic effects of angiotensin-converting enzyme inhib-itors and angiotensin receptor blockers. The hemodynamicload of pregnancy and delivery, coupled with the prolongeddiscontinuation of optimal medical therapy during preg-nancy, often because of contraindications or patient prefer-






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ence, might also predispose to further late negative effectson ventricular function (4,51).

Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy is an autosomal dominantdisease due to mutations of the cardiac sarcomere proteins. This type of cardiomyopathy has a phenotypically variablecourse and is diagnosed with a frequency of approximately 1in 500 of the adult population (52,53). Morphological andfunctional features include asymmetric hypertrophy of theleft ventricle, a nondilated left ventricular cavity, and pre-served systolic function with impaired diastolic function(Fig. 3) (54–57), whereas myobrillary disarray is thehallmark pathologic feature. Although no evidence of ob-struction is present in the majority of patients with HCM,a left ventricular outow tract (LVOT) obstruction ispresent at rest in approximately 20% of patients (58). Left ventricular outow tract obstruction is directly related to theseverity of hypertrophy of the basal septum, which obstructsthe contours of the LVOT. Secondary causes of dynamicoutow obstruction occur as the result of a venturi effect inthe narrowed outow tract, causing systolic anterior motionof the mitral valve (52). Diastolic dysfunction is frequently present due to impaired relaxation of the left ventricle. Bothatrial and ventricular arrhythmias can coexist with HCM,leading to palpitations and sudden cardiac death as well asembolic stroke, worsening diastolic heart failure, and dys-pnea. With the increased use of echocardiography andgenetic and family screening, more women of child-bearingage are being diagnosed with HCM.Screening. The clinical diagnosis of HCM is usually accomplished with an integration of history, physicalexamination, electrocardiography, and echocardiography. There is phenotypic heterogeneity of patients withHCM, because the distribution of left ventricular hyper-trophy is inconsistent, with no single morphologic ex-pression considered to be “typical.” Anteroseptal hyper-trophy is most frequently involved, whereas patterns suchas concentric hypertrophy or pure apical involvement areless common (59). Echocardiography typically demon-strates a hypertrophied, nondilated left ventricle with anobserved wall thickness of 15 mm, in the absence of any other cardiac or systemic disease known to cause in-creased wall thickness. Left ventricular outow tractobstruction is assessed by the measured gradient asestimated by Doppler ow analysis and the degree of systolic anterior motion of the mitral valve. A Doppler-derived pressure gradient of 30 mm Hg or more usually correlates with symptoms (53). Mitral regurgitation iscommonly observed by echocardiography and is due tomitral valve abnormalities related to systolic anteriormotion of the mitral valve (Fig. 3).

Although screening for the usual echocardiographic cri-teria for HCM is commonplace, the landscape is changing with regard to clinical evaluation and screening for this

disease process and might involve routine genetic screeningin the near future. To date, molecular genetics have iden-tied at least 11 individual genes found to be responsible for

Figure 3 2-Dimensional Transthoracic

Echocardiography in HCM

(A) Parasternal long-axis view of a hypertrophied, nondilated left ventricle (LV).Septal wall thickness is 15 mm. (B) Doppler ow analysis of LV outow tract.A resting gradient of 39 mm Hg increases to 50 mm Hg with Valsalva maneuver.The increased blood volume as pregnancy progresses can allow for a normaliza-tion of the nondilated LV in hypertrophic cardiomyopathy (HCM), which often less-ens the degree of obstruction in the left ventricular cavity. However, if mitralregurgitation is present, the increase in blood volume can worsen the degree of regurgitation as pregnancy progresses. Mitral regurgitation, even if severe, is oftenwell-tolerated in pregnancy due to the reduced systemic vascular resistancealready present in pregnancy and low resistance circuit of the placenta. IVSinterventricular septum; LVPW left ventricular posterior wall; RV right ventricle.





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a hypertrophic phenotype, representing several hundredindividual mutations within genes coding for various sarco-meric proteins, the most common of which are beta-myosinheavy chain and myosin binding protein C (60,61). Withthe identication of causal genes came the understandingthat one-half of these cases are familial, whereas the othersare felt to be due to sporadic mutations. Familial casesexhibit classic Mendelian genetics and are transmitted in anautosomal dominant fashion. Despite our increased under-standing of this disease, a denitive genetic causality can befound in only 40% of cases. Moreover, there are likely many more responsible mutations yet to be identied, and ourunderstanding of the phenotypical expression of these genedefects is also changing (62). Notably, among patients withseemingly identical genotypes, the expression of these mu-tations is remarkably variable. Some genetically prone indi- viduals might not manifest phenotypically identiable dis-ease until later in adult life (63). Such implications areimportant in the family planning of any patient with HCM, where there is a 50% chance of producing an affected child.Counseling should include the need for genetic screening of known mutations and, potentially, for lifelong clinical andechocardiographic follow-up.Risks in pregnancy. The risks of HCM and pregnancy aredue to hemodynamic deterioration, arrhythmias, or suddencardiac death. Most women with HCM who experience noor mild symptoms before pregnancy will likely toleratepregnancy well (52,53,64). The increased blood volume andstroke volume of pregnancy usually result in a mild dilationof the left ventricular end diastolic dimension and, thus,lowering of the dynamic outow tract gradient. However, asmall subset of patients, with moderate or severe symptomsbefore pregnancy, are at increased risk for cardiac events. The higher the LVOT gradient is before pregnancy orduring the rst trimester, the higher the likelihood thatsymptoms will progress. Moreover, the subset of patients with severe LVOT obstruction (those with a gradient of

100 mm Hg) are at the highest risk of hemodynamicdeterioration during pregnancy (52).

Sudden death is a recognized complication of HCMduring pregnancy, particularly in patients with severe out-ow tract obstruction or in those with other signicant risk factors for sudden cardiac death (53). Major risk factorsinclude previous out-of-hospital arrest or documented sus-tained ventricular tachycardia, or a strong family history of HCM with associated sudden death. Minor risk factors forsudden death include severe septal hypertrophy (3 cm),nonsustained ventricular tachycardia on Holter monitoring,and a decrease in blood pressure with exercise.Management in pregnancy. Although most patients withHCM are asymptomatic, common symptoms that women with HCM experience during pregnancy include exertionalchest pain, fatigue, dyspnea, palpitations, and syncope. When these symptoms are reported during pregnancy,however, most women note experiencing similar symptomsbefore pregnancy as well (64). Sudden death might also be

the initial presentation of undiagnosed disease. Dyspnea onexertion is the most common symptom, mainly owing todiastolic dysfunction and outow tract obstruction. As aresult, there is a potential for clinical heart failure amongpregnant women (53). However, for the majority of pa-tients, the hemodynamic changes that occur are usually well-tolerated in pregnancy. Although the overall decreasein systemic vascular resistance during pregnancy might bedetrimental in HCM patients, this is usually offset by theincrease in intravascular volume. Although HCM patientsare sensitive to alterations of intravascular volume andcardiac rate and rhythm, the increase in intravascular vol-ume and stroke volume actually reduces LVOT obstruction.Diastolic dysfunction is common in HCM patients who aremore likely to develop pulmonary edema, aggravated by mitral regurgitation, which occurs secondary to systolicanterior motion of the mitral valve (65).

If symptoms of heart failure develop during pregnancy, itis indicative of poor functional class before pregnancy,because clinical deterioration is twice as likely to occur inpatients with antenatal outow obstruction as in those without. The higher incidence of heart failure in somepatients has been attributed to the genetic heterogeneity of the disease (66). Most women who progress to NYHAfunctional class III or IV heart failure were previously symptomatic, just as patients who experienced atrial bril-lation or syncope experienced similar symptoms beforepregnancy. Thus, despite a reduced left ventricular cavity size and poor left ventricular compliance, women withHCM seem to be able to tolerate the hemodynamic burdensassociated with pregnancy. Studies have suggested a similarincidence of cardiac events between nonpregnant and preg-nant patients, suggesting that pregnancy does not alter theclinical course of women with HCM.

There are few available data describing the optimalmanagement of pregnant women with HCM. Table 5 offerssome general considerations for management. Studies by Thaman et al. (64) and Autore et al. (53) show thatapproximately one-quarter to one-third of pregnant patients were receiving medications for symptomatic HCM—beta-blockers being the most common. Although beta-blockerand calcium-channel blocker use has been described as safein this population, with little effect on fetal outcome, someof the women discontinued these medications during preg-nancy due to the potential for adverse fetal events. In women who received medications before pregnancy, discon-tinuing medications before or early in pregnancy was notshown to precipitate signicant clinical deterioration (64).

In a study by Autore et al. (53), the maternal mortality forpatients with HCM was increased compared with that of the general population. The absolute maternal mortality waslow, however, and seemed to be principally conned to women at particularly high risk. Two maternal mortalities were described in 100 women over the course of 199 livebirths. Both women died suddenly, and both were known tobe symptomatic before pregnancy. In the presence of a






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favorable risk prole as assessed clinically, Autore et al. (53)found that the progression of symptoms, the developmentof atrial brillation, and syncope were uncommon duringpregnancy. In another study by Thaman et al. (64) of 127 women and 271 pregnancies, the overall complication rate was low, and pregnancy was tolerated well. Approximately 28% of women reported cardiac symptoms during preg-nancy, with most of them reporting similar symptomsbefore pregnancy. In this group, there was no symptomaticdeterioration. Two patients developed clinical heart failureduring the post-partum period, but there were no maternaldeaths. Unfortunately, there were 3 unexplained intrauter-ine deaths, all occurring in women taking cardiac medica-tions. Moreover, in a retrospective study of 41 women overthe course of 132 pregnancies, there were no maternaldeaths and no aggravation of symptoms (67). Therefore,there seems to be a subset of patients who are at higher risk of sudden death or heart failure, who would potentially benet from aggressive risk stratication. Thus, althoughmost women with HCM can safely tolerate pregnancy, it isimportant to have an appreciation of the underlying pathol-ogy and to identify those at particularly high risk whenmanaging these patients.Management during labor and delivery. Although preg-nancy seems to be well-tolerated in HCM, the physiologicchanges that occur during labor and delivery seem to bemore problematic. During labor, a catecholamine-inducedincrease in heart rate and stroke volume increase cardiacoutput. As noted previously, tachycardia shortens the left ventricular diastolic lling period, decreasing preload, andexacerbates outow obstruction. Moreover, performance of the Valsalva maneuver during labor and delivery, whichhelps the fetus descend during the second stage of labor, canalso impair venous return and impairs cardiac output inpatients with signicant obstruction (68). A similar problemcan occur in the setting of substantial blood loss during orafter delivery (69).

It is generally accepted that patients with HCM shoulddeliver at a high-risk center, with expertise in the manage-ment of this condition. Beta-blockers are particularly usefulduring labor and delivery, because these agents blunt the

normal increases in heart rate and contractility, allowing foradequate diastolic lling, which might decrease the outow tract obstruction (66). Low-dose diuretics might be neces-sary if pulmonary edema develops, but aggressive diuresiscan result in a reduction of intravascular volume and bloodpressure, leading to a reduction in preload and potential worsening LVOT obstruction (52). Because of the sensitiv-ity of HCM patients to disturbances in intravascular volumeand blood pressure, epidural anesthesia should be avoideddue to the potential for hypotension, but vaginal deliveries with regional anesthesia have been documented. Likewise,the use of prostaglandins for the induction of labor is notadvised, secondary to inherent vasodilatory effects. Normal vaginal delivery is considered safe, whereas cesarean sectionis reserved for emergency situations (69). In the U.K.registry of high-risk obstetric anesthesia between the yearsof 1997 and 2001, there were 26 patients with an underlyingcardiomyopathy. Of those with HCM, 75% of these pa-tients were delivered by caesarian section and receivedgeneral anesthesia (70). Most women received generalanesthesia out of concern for a rapid fall in systemic vascularresistance in patients with a xed cardiac output due to thedegree of outow stenosis. Vasopressor agents and uidscan be given to treat hypotension, but it is preferable to usea pure alpha-agonist such as phenylephrine, because it lacksany inotropic properties (68). In HCM, inotropes can worsen the outow gradient and are relatively contraindi-cated (51).

Conclusions

In women with underlying cardiomyopathy, changes inintravascular volume, cardiac output, and peripheral vascularresistance coupled with an impaired ventricular reserve poseunique challenges in the management of pregnancy, labor,and delivery. Pre-conception risk assessment and propercounseling with regard to the safety of pregnancy is crucial,but more data are necessary to provide more informedrecommendations to expectant mothers. Pre-conceptioncounseling offers information regarding the risks of preg-nancy to both the mother and the fetus and should bedelivered by practitioners with considerable experience inthis area. It is imperative to assess baseline functional statusand allow early referral to a cardiologist for medical opti-mization to better prepare for the difculties that pregnancy,labor, and delivery pose to this unique population.

Reprint requests and correspondence: Dr. Kathleen Stergiopoulos,Department of Medicine, Division of Cardiovascular Disease, HSC T-16 080, Stony Brook University Medical Center, Stony Brook,New York 11974-8167. Email: [email protected].

REFERENCES

1. Silversides CK, Coleman JM. Physiologic changes in pregnancy. In:Oakley C, Warnes CA, editors. Heart Disease in Pregnancy. Malden,MA: Blackwell Publishing, 2007:173–85.

General Considerations for Management of Pregnant Women With Hypertrophic CardiomyopathyTable 5 General Considerations for Management of Pregnant Women With Hypertrophic Cardiomyopathy

Assessment of symptoms and functional status before pregnancy

Determine the degree of LVOT obstruction at rest and with Valsalva maneuver(by echocardiogram) to identify those with severe obstruction

Risk stratication of sudden cardiac death

Institute medical therapy for symptomatic patients with beta-blockade(verapamil as an alternative)*

Avoidance of decrease in preload (straining, dehydration, diureticsonly if needed)

Left lateral decubitus position

Avoid inotropes and vasodilators

For the hypotensive patient, balance uids and vasopressor agents

*Verapamil is not considered safe in patients with severe resting left ventricular outow tract(LVOT) obstruction.



mailto:[email protected]





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Key Words: cardiomyopathy y dilated cardiomyopathy y hypertrophiccardiomyopathy y pregnancy.





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doi:10.1016/j.jacc.2011.04.0142011;58;337-350 J. Am. Coll. Cardiol.

Kathleen Stergiopoulos, Elaine Shiang, and Travis BenchPregnancy in Patients With Pre-Existing Cardiomyopathies

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